Cardiomyopathies in China: A 2018–2019 state‐of‐the‐art review

Abstract

Cardiomyopathies are diseases of the cardiac muscle and are often characterized by ventricular dilation, hypertrophy, and cardiac arrhythmia. Patients with cardiomyopathies often experience sudden death and cardiac failure and require cardiac transplantation during the course of disease progression. Early diagnosis, differential diagnosis, and genetic consultation depend on imaging techniques, genetic testing, and new emerging diagnostic tools such as serum biomarkers. The molecular genetics of cardiomyopathies has been widely studied recently. The discovery of mechanisms underlying heterogeneity and overlapping of the phenotypes of cardiomyopathies has revealed the existence of disease modifiers, and this has led to the emergence of novel disease-modifying therapy. This 2018–2019 state-of-the-art review outlines the pathogenesis, diagnosis, and treatment of cardiomyopathies in China.

Inflammation and cardiovascular diseases

“…Along with the recognition of inflammation as a major factor that modulates HF, the role of inflammatory processes in the pathogenesis of cardiomyopathy has also become widely accepted. An up-to-date review by Tian et al 17 provided a framework to deepen our understanding of the genetic basis, pathogenetic phases, and clinical faces of cardiomyopathy, including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ACM), and restrictive cardiomyopathy.…”

Effect of Mavacamten on Chinese Patients With Symptomatic Obstructive Hypertrophic Cardiomyopathy

“…This leads to increased systolic anterior motion of the mitral valve, leading to mitral valve–ventricular septal contact, which contributes to a pressure gradient between the LV chamber and systemic circulation . Current standard pharmacologic therapies for oHCM, such as β-blockers, nondihydropyridine calcium channel blockers, and disopyramide, may offer symptomatic relief but are nonspecific and do not address the underlying pathophysiological mechanisms behind HCM nor alter the disease course . For severe oHCM that is refractory to pharmacologic treatment, septal reduction therapy (SRT) is effective in relieving oHCM symptoms .…”

“… 6 , 7 , 8 Current standard pharmacologic therapies for oHCM, such as β-blockers, nondihydropyridine calcium channel blockers, and disopyramide, may offer symptomatic relief but are nonspecific and do not address the underlying pathophysiological mechanisms behind HCM nor alter the disease course. 3 , 8 , 10 , 11 , 12 For severe oHCM that is refractory to pharmacologic treatment, septal reduction therapy (SRT) is effective in relieving oHCM symptoms. 3 , 8 However, such invasive procedures carry inherent surgical risks and demand expertise that is not widely accessible in China and other countries.…”

Study design and rationale of EXPLORER-CN: a phase III, randomised, double-blind, placebo-controlled clinical study to evaluate the efficacy and safety of mavacamten in Chinese adults with symptomatic obstructive hypertrophic cardiomyopathy

“…The treatment pattern of oHCM is similar in both China and other countries 6–8. Standard pharmacological options for oHCM are β-blockers, non-dihydropyridine calcium-channel blockers (eg, verapamil, diltiazem)6 7 9 10 or disopyramide in combination with either class of drugs 6 7 9. In patients with refractory oHCM, septal reduction therapy using surgical myectomy or percutaneous alcohol septal ablation is considered safe and effective in relieving LVOTO 6–10.…”

“…Standard pharmacological options for oHCM are β-blockers, non-dihydropyridine calcium-channel blockers (eg, verapamil, diltiazem)6 7 9 10 or disopyramide in combination with either class of drugs 6 7 9. In patients with refractory oHCM, septal reduction therapy using surgical myectomy or percutaneous alcohol septal ablation is considered safe and effective in relieving LVOTO 6–10. However, the risks of cardiac surgery, lack of experienced centres and the fact that many patients are not amenable to septal reduction, has highlighted the need for alternative approaches 8 11.…”